A tale of two patients with Mendelian hypertension.

Conn emphasized the triad of hypertension, hypokalemia, and metabolic alkalosis in his seminal account of patients with primary aldosteronism.1 He believed that primary aldosteronism was a very common cause of hypertension. Kaplan introduced the discussion as to the actual prevalence of primary aldosteronism, a topic still debated today.2,3 Suffice it to say that hypokalemic hypertension is a fixed entity in the minds of clinicians as synonymous with primary aldosteronism, and perhaps this reaction is appropriate. Nonetheless, there are other diagnostic considerations. For instance, licorice gluttony looks exactly like primary aldosteronism clinically; the diagnosis requires a particularly high grade of detective work.4 However, Mendelian disorders have become increasingly recognized, especially because elucidation of their molecular mechanisms provides reliable diagnostic tools.5 Another confounder is the fact that contrary to prevailing clinical opinion, electrolyte and acid-base abnormalities are absent in many patients with primary aldosteronism.6 We describe 2 remarkable patients who were clinical adventures for us and provided several important lessons.